Kennedy syndrome - Altmeyers Encyclopedia

Definition
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X-linked recessive inherited disease caused by a dysfunction of the androgen receptor

Manifestation
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Only men are affected, women act as conductors. Manifestation between the ages of 20 and 40.

Clinic
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Stress-related muscle cramps and fasciculations, then proximal muscle weakness, slow progression with development of bulbar symptoms (dysarthria, dysphagia). In 50% of cases gynecomastia, testicular atrophy with restriction of spermatogenesis and secondary impotentia gestandi; frequent increase of LH and FSH, only in one third decrease of serum testosterone. Commonly diabetes mellitus or subclinical glucose tolerance disorder.